Skip to main content
Log in

Ceramide hexosides in niemann-pick disease brain

  • Short Communications
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Zusammenfassung

In normalem Gehirngewebe und in Gehirngewebe einer Niemann-Pickschen Erkrankung vom Typus A wurden Glykosphingolipide unter Verwendung der Dünnschichtchromatographie und der Flüssig-Gaschromatographie bestimmt. Bei der Niemann-Pickschen Erkrankung fand sich eine Zunahme der Ceramid-Trihexoside, die durch Digalactosylglucosylceramide bedingt war. Auch das Glucosylcerebrosid war vermehrt.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

References

  • Bartlett, G. R.: Phosphorous assay in column chromatography. J. biol. Chem. 234, 466–468 (1959)

    Google Scholar 

  • Brunngraber, E. G., Berra, B., Zambotti, V.: Altered levels of tissue glycoproteins, gangliosides, glycosaminoglycans and lipids in Niemann-Pick disease. Clin. chim. Acta 48, 173–181 (1973)

    Google Scholar 

  • Cummings, J. N.: Abnormalities in lipid metabolism in two members of a family with Niemann-Pick disease. In: S. M. Aronson, B. W. Volk, Cerebral sphingolipidoses, pp. 171–178. New York: Academic Press 1962

    Google Scholar 

  • Dawson, G.: Glycosphingolipid levels in an unusual neurovisceral storage disease characterized by lactosylceramide galactosyl hydrolase deficiency: lactosylceramidosis. J. Lipid Res. 13, 207–219 (1972)

    Google Scholar 

  • Fredrickson, D. S., Sloan, H. R.: Sphingomyelin lipidoses: Niemann-Pick disease. In: J. B. Stanbury, J. B. Wyngarden, D. S. Fredrickson, The metabolic basis of inherited disease, 3rd ed., pp. 783–807. New York: McGraw Hill 1972

    Google Scholar 

  • Folch, J., Lees, M., Sloan-Stanley, G. H.: A simple method for the isolation and purification of total lipides from animal tissue. J. biol. Chem. 226, 497–507 (1957)

    Google Scholar 

  • Hoffman, L. M., Schneck, L.: Sphingolipid analysis. In: B. W. Volk, L. Schneck, The gangliosidoses, pp. 223–231. New York: Plenum 1975

    Google Scholar 

  • Hoshi, M., Williams, M., Kishimoto, Y.: Characterization of brain cerebrosides at early stages of development in the rat. J. Neurochem. 21, 709–712 (1973)

    Google Scholar 

  • Kanfer, J. N.: Preparation of gangliosides. In: J. M. Lowenstein, Methods in enzymology, Vol. XIV, pp. 660–665. New York-London: Academic Press 1969

    Google Scholar 

  • Kannan, R., Tjiong, H., Debuch, H., Wiedemann, H.: Unusual glycolipids in brain cortex of a visceral lipidosis (Niemann-Pick disease?). Hoppe-Seylers Z. physiol. Chem. 355, 551–556 (1974)

    Google Scholar 

  • Leeden, R., Yu, R. K.: Isolation and purification of gangliosides. In: S. Bogoch, Biological diagnosis of brain disorders, pp. 372–376. New York: Spectrum 1973

    Google Scholar 

  • Rouser, G., Kritchevsky, G., Yamamoto, A.: Column chromatographic and associated procedures for separation and determination of phosphatides and glycolipids. In: G. Marinetti, Lipid chromatographic analysis, Vol. 7, pp. 99–162. New York: Marcel Dekker 1967

    Google Scholar 

  • Sandhoff, K., Harper, K., Wassle, W., Jatzkewitz, H.: Enzyme alterations and lipid storage in three variants of Tay-Sachs disease. J. Neurochem. 18, 2469–2489 (1971)

    Google Scholar 

  • Schneck, L., Pinkett, B., Volk, B. W.: Asialo-GM2 ganglioside in fetal Tay-Sachs disease brain. J. Neurochem. 24, 183–184 (1975)

    Google Scholar 

  • Smid, F., Reinisova, J.: A densitometric method for the determination of gangliosides after their separation by thin layer chromatography and detection with resorcinol reagent. J. Chromatogr. 86, 200–204 (1973)

    Google Scholar 

  • Suzuki, K.: The pattern of mammalian brain gangliosides. II. Evaluation of the extraction procedures, post mortem changes and the effect of formalin preservation. J. Neurochem. 12, 629–638 (1965)

    Google Scholar 

  • Suzuki, K., Suzuki, R., Kamoshita, S.: Chemical pathology of GM1-gangliosidosis (generalized gangliosidosis). J. Neuropath. exp. Neurol. 28, 25–73 (1969)

    Google Scholar 

  • Svennerholm, L.: The quantitative estimation of cerebrosides in nervous tissue. J. Neurochem. 1, 42–53 (1956)

    Google Scholar 

  • Svennerholm, L.: Quantitative estimation of sialic acid. II. A colorimetric resorcinol-hydrochloric acid method. Biochim. biophys. Acta (Amst.) 24, 604–611 (1957)

    Google Scholar 

  • Vance, D., Sweeley, C. C.: Quantitative determination of the neutral glycosyl ceramides in blood. J. Lipid Res. 8, 621–630 (1967)

    Google Scholar 

  • Wenger, D. A., Okada, S., O'Brien, J. S.: Studies on the substrate specificity of hexosaminidase A and B from liver. Arch. Biochem. Biophys. 153, 116–129 (1972)

    Google Scholar 

  • Zalc, B., Pollet, S. A., Harpin, M. L., Baumann, N. A.: Ceramide biosynthesis in mouse brain microsomes: comparison between C57/BL controls and quaking mutants. Brain Res. 81, 511–518 (1974)

    Google Scholar 

  • Zalkis, A., Zak, B., Boyle, A. J.: New method for direct determination of serum cholesterol. J. Lab. clin. Med. 41, 486–492 (1953)

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Greenbaum, M., Hoffman, L.M. & Schneck, L. Ceramide hexosides in niemann-pick disease brain. J. Neurol. 213, 251–255 (1976). https://doi.org/10.1007/BF00312874

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00312874

Key words

Navigation